An unusual occurrence of autoimmune pancreatitis after gam-Covid-Vac (Sputnik V): A case report and literature review.

The safety profile of the Sputnik V vaccine is generally reassuring. Nevertheless, an enhanced risk of new-onset of immune-mediated diseases has been increasingly reported following the adenoviral-based Covid-19 vaccine, including inflammatory arthritis, Guillain-Barré syndrome, optical neuromyelitis, acute disseminated encephalomyelitis, subacute thyroiditis and acute liver injury as well as glomerulopathy. However, no case of autoimmune pancreatitis has been reported yet. Herein, we describe a case of type I autoimmune pancreatitis that may be due to the Sputnik V Covid-19 vaccine.

A case of type 2 autoimmune pancreatitis with spontaneous remission.

A 70-year-old man with epigastric pain was referred to our hospital. Computed tomography and magnetic resonance imaging showed the diffusely enlarged pancreas compared to his normal pancreas 6 months prior to presentation. Serum levels of IgG4 and amylase were normal, while C-reactive protein was slightly elevated. Endoscopic ultrasound-guided fine-needle biopsy of the pancreas revealed acinar-ductal metaplasia with neutrophil infiltration and without infiltration of IgG4-positive plasma cells. After the clinical diagnosis of type 2 autoimmune pancreatitis (AIP), his symptoms spontaneously improved without steroid therapy. Three months later, radiological findings showed improved pancreas size and serological findings. The pathological diagnosis of type 2 AIP using endoscopic ultrasound-guided fine-needle biopsy is challenging, particularly for proving granulocyte epithelial lesions. This was a valuable type 2 AIP case in which the images before, at the time of onset, and at the time of spontaneous remission were evaluated.

Post-COVID-19 polyautoimmunity - Fact or coincidence: A case report.

COVID-19 exhibits diverse and systemic clinical symptoms, much like systemic autoimmune diseases, and there are notable similarities in the immune responses seen in both conditions. There are rare reports of ulcerative colitis and autoimmune hepatitis triggered by COVID-19 infection. Reported herein is a case of a previously healthy patient who was diagnosed with chronic colitis resembling ulcerative colitis, autoimmune pancreatitis, and suspected immune-mediated hepatitis (AIH-like hepatitis) 2 months after a COVID-19 infection. A 33-year-old COVID-19-vaccinated male, presented with abdominal pain, nausea, and vomiting for 2 days. He also had bloody diarrhea that persisted for 2 months after recovering from a COVID-19 infection. A diagnosis of acute pancreatitis was confirmed by markedly elevated serum amylase and lipase and a CT scan of the abdomen. Colonoscopy and histopathology findings also confirmed a diagnosis of chronic colitis resembling ulcerative colitis (Mayo Endoscopy Subscore 3). Marked improvement in bloody diarrhea was observed within 72 h of treatment with IV prednisolone. MRI of the abdomen performed due to an unresolved clinical picture of pancreatitis revealed a bulky pancreas showing delayed diffuse homogenous enhancement, findings possibly consistent with autoimmune pancreatitis. Investigation for elevated liver transaminases showed high titers of antinuclear antibodies and anti-smooth muscle (anti-actin) antibodies while viral hepatitis markers were negative. The patient had already been started on steroid therapy before the lab results were available, with rapid normalization of liver enzymes following treatment. A liver biopsy was not performed. The patient is currently on mesalazine 4 gr/day, and azathioprine 100 mg/day - oral steroids had been tapered and discontinued. Seven months after the initial diagnosis, the patient remains symptom-free. A high level of suspicion for autoimmune disorders is required when assessing patients with a history of COVID-19 infection, although diagnostic pathways remain the same, with generally good response and remission rates to conventional treatment.

Type 1 Autoimmune Pancreatitis Unmasked by COVID-19 Vaccine.

Autoimmune pancreatitis is a rare fibro-inflammatory disease with 2 distinct subtypes of which each has their own clinical presentation, risk factors, and histopathological patterns. We present a case of newly diagnosed type 1 autoimmune pancreatitis in a symptomatic 54-year-old man with stable ulcerative colitis 1 month after COVID-19 vaccination. Previous reports have indicated that vaccinations can trigger autoimmune disease in predisposed individuals. This case discusses the occurrence of autoimmune pancreatitis triggered after COVID-19 vaccination.

A PROBABLE CASE OF IGG4-RELATED LUNG DISEASE MIMICKING PNEUMOCONIOSIS

SESSION TITLE: Autoimmune Diseases Gone Wild: Rare Cases of Pulmonary Manifestations SESSION TYPE: Rapid Fire Case Reports PRESENTED ON: 10/18/2022 01:35 pm - 02:35 pm INTRODUCTION: Immunoglobulin G4-related disease (IgG4-RD) is a complex entity related to autoimmune dysfunction and inflammation that can cause mass-like lesions and fibrosis of a variety of organs, including pancreas and/or lungs. IgG4-RD in the lung can have diverse clinical and radiographic presentations. We present a case of suspected IgG4-RD that manifested as idiopathic pancreatitis and interstitial lung disease that mimicked coal workers' pneumoconiosis. CASE PRESENTATION: A 72 year-old male with a decades-long coal mining history and a presumptive diagnosis of coal-worker's pneumoconiosis was admitted to the hospital for necrotizing pancreatitis. There was no evidence of gallstones, elevated triglycerides, history of alcohol use or medication known to precipitate pancreatitis. Two years prior, a presumptive diagnosis of coal-worker's pneumoconiosis had been reached largely on the basis of history and chest imaging (Figure 1) showing a progressive massive pulmonary fibrosis pattern. His hospital course was protracted and complicated by nosocomial COVID-19 treated with remdesivir and a 10-day course of dexamethasone. He then had persistent hypoxemia that worsened after dexamethasone was discontinued. Empiric high-dose methylprednisolone was given and the hypoxemia improved dramatically. However, the hypoxemia and pancreatitis repeatedly worsened with significant dose decrease. Inpatient CT chest showed worsening interstitial reticulation and ground-glass opacities superimposed on prior fibrosis (Figure 2). Serum IgG subclass levels were checked;IgG4 and IgG4:IgG ratio were mildly elevated at 93mg/dL and 0.09, respectively. In the setting of idiopathic pancreatitis, pulmonary fibrosis, and steroid-sensitive hypoxemia, he was diagnosed with probable IgG4-RD involving pancreas and lungs. An association between inhaled occupational exposures and development of IgG4-RD has been observed. To confirm the diagnosis of pulmonary IgG4-RD, a tissue biopsy will be necessary. He is now discharged from hospital on a long steroid taper. DISCUSSION: A serum IgG4 level >125mg/dL or an IgG4:total IgG ratio >0.08 support the diagnosis, as does clinical response to steroids. However, these criteria are nonspecific and will be in the normal range in a substantial minority of cases. Lymphocytes and a predominance of IgG4-positive plasma cells infiltrating fibrotic tissue in involved organs are pathologic hallmarks of IgG4-RD. Lung involvement in patients with pancreatitis due to IgG4-RD is common and likely under recognized. CONCLUSIONS: Pulmonary involvement in IgG4-RD can show a wide array of radiographic patterns, but that seen in this case with pseudotumor and fibrosis is among the most commonly reported. Given the overlap in risk factors and radiographic appearance between IgG4-RD and pneumoconiosis, vigilance for IgG4-RD is warranted. Reference #1: Hirano K., Kawabe T., Komatsu Y., et al. High-rate pulmonary involvement in autoimmune pancreatitis. Internal Medicine Journal. 2006;36(1):58–61. doi: 10.1111/j.1445-5994.2006.01009.x Reference #2: Kamisawa T, Zen Y, Pillai S, Stone JH. IgG4-related disease. Lancet. 2015 Apr 11;385(9976):1460-71. doi: 10.1016/S0140-6736(14)60720-0. Epub 2014 Dec 4. PMID: 25481618. Reference #3: de Buy Wenniger, L. J., Culver, E. L., & Beuers, U. (2014). Exposure to occupational antigens might predispose to IgG4-related disease. Hepatology (Baltimore, Md.), 60(4), 1453–1454. https://doi.org/10.1002/hep.26999 DISCLOSURES: No relevant relationships by Jordan Minish, source=Web Response No relevant relationships by Robert Ousley, source=Web Response No relevant relationships by Meagan Reif, source=Web Response No relevant relationships by Derek Russell, source=Web Response

Acute liver injury and IgG4-related autoimmune pancreatitis following mRNA-based COVID-19 vaccination.

IgG4-related disease (IgG4-RD) is a fibro-inflammatory disease that can affect multiple organs. Autoimmune pancreatitis type 1 is a manifestation of IgG4-RD and can often mimic tumor-like masses. Autoimmune phenomena following COVID-19 mRNA vaccination are being increasingly reported. Currently, there are no cases in which IgG4-RD involving the hepatobiliary system has been reported following the COVID-19 vaccination. We present the first case of IgG4-RD and AIP type 1 to be associated with the mRNA-based COVID-19 vaccination.

POS-534 AUTOIMMUNE PANCREATITIS IN SYSTEMIC LUPUS ERYTHEMATOSUS: CASE REPORT

Introduction: Systemic lupus erythematosus (SLE) is a chronic, multifaceted autoimmune inflammatory disease with a wide range of clinical presentations resulting from its effect on multiple organ systems. We report a case of SLE associated with autoimmune pancreatitis. Methods: In this study, we present a patient diagnosed as having SLE who developed acute auto-immune pancreatitis. Results: This is a 36-year-old woman, with lupus diagnosed since 2009. Initially, the manifestations of her disease were dermatological and articular. Then appeared the renal involvement with a lupus nephropathy class IV at the renal biopsy (PBR). She was previously treated with the NIH protocol then oral prednisolone with improvement in her symptoms. She continued these medications but was lost to follow-up since 2016 and presented after 6 years with pigmented skin lesions on her upper and lower limbs, abdominal pain and distension, vomiting, and an altered general condition. In biology, the patient presented a functional acute kidney failure, an elevated amylasemia (30 times normal), an elevated lipasemia (6 times normal), a normocytic normochromic hemolytic anemia with positive direct coombs test, lymphopenia, a positive immunological assessment (AAN, anti DNA AC, anti Sm, anti SSA, anti RNP), a low C3, a low C4. The patient presented a lupus flare with a SLEDAI score of 6 points: moderate lupus activity. Ultrasound confirmed a large abundance of ascites. Ascites fluid puncture showed an exudate with hyperleukocytosis with predominantly PNN and no germ on direct examination nor on culture.The infectious origin of the pancreatitis was eliminated (CMV, tuberculosis, covid19), as well as the tumoral origin (negative tumor markers, abdominal CT scan showed a swollen pancreas in its caudal portion with loss of physiological lobulations and normal spontaneous density.Necrosis flows difficult to individualize. In addition, no deep neoplastic focus). The autoimmune origin of the pancreatitis due to its lupus attack was retained. She was put on corticosteroids (500mg intravenously for 3 days) then relayed by oral route, albumin infusion, evacuation puncture. The subsequent evolution was marked by the progressive normalization of the pancreatic balance and the slower disappearance of the ascites. Conclusions: Acute pancreatitis is an unusual manifestation of SLE and it should be suspected in any SLE patient with these similar symptoms. In many cases, this complication has been attributed to the drugs administered. In our case, a favorable course of pancreatitis with corticosteroids adds further evidence to the idea that lupus-related pancreatitis is not a side effect of corticosteroid therapy. Moreover, treatment with these medications improves the prognosis. No conflict of interest

Unusual neurological manifestations in children during the COVID-19 pandemic

Objective: To present paediatric cases of unusual neuroinflammatory conditions encountered during the COVID-19 pandemic in Trinidad & Tobago. Methods: Retrospective study design. Inpatient paediatric patients (aged 0-16 years) hospitalized for neurological complaints from June 2020 - August 2021 at EWMSC. Outcome measures were age at presentation, sex, ethnicity, diagnosis, radiological findings, blood/CSF findings, COVID-19 PCR and antibodies testing, treatment, outcomes and other systems involved. Results: Twenty (20) patients (aged 4-months-old to 15-years-old) had documented neurological involvement. 50% had a diagnosis of ADEM/ADS/AHNE;45% had a diagnosis of either CNS vasculitis (n=3), autoimmune encephalitis (n=3) or GBS (n=3);5% had a diagnosis of acute COVID-19 encephalitis. 70% were of African descent. The youngest age group (0-4 years) (n=11) constituted more males (82%) whereas the eldest age group (10-15 years) (n=3) were all females. Neuroimaging findings were corpus callosal lesions;deep white matter T2 hyperintensities;cerebellar involvement;area postrema and brainstem/C-spine involvement;microhaemorrhages and necrotizing/haemorrhagic lesions (peripheral/central). 70% of patients were either SARS-CoV-2 PCR or COVID-19 antibodies positive. Other systems were involved in 40% to 62.5% (n=5) had cardiac involvement (myocarditis, coronary arteries dilatation, valve regurgitation) and 37.5% (n=3) had pancreatic involvement (autoimmune pancreatitis, type 1 diabetes mellitus). Treatment modalities for CNS manifestations (n=17) were clinically based - 24% (n=4) 3rd line treatment, 29% (n=5) 2nd line treatment, 41% (n=7) 1st line treatment and 6% (n=1) requiring no treatment. All 3 patients with a diagnosis of GBS responded appropriately to IVIG. Developmental outcomes were worst in patients with a diagnosis of autoimmune encephalitis. Conclusion: We have had an explosion of neuro-inflammatory cases since the COVID-19 pandemic began. The range of neuroradiological diagnoses and other systemic involvement (including criteria for PIMS) are interesting, alluding to a neuroinflammatory mechanism. Effects on long-term sequelae and developmental outcomes are concerning in some cases, however, still unknown at this stage.

COVID-19 and the biliary tree: Atypicall manifestations of disease during the pandemic

Case report-IntroductionCOVID-19, the infectious disease caused by the novel coronavirus SARS-CoV-2, and first described in Wuhan, China in December 2019, has affected more than 19 million patients worldwide and resulted in more than 700,000 deaths at the time of writing1. Patients with rheumatic diseases and those receiving immunosuppressive treatment are felt to be at greater risk of complications from this illness, though registry and trial data should help refine our understanding of these risks. We hereby describe a case of COVID-19 complicating an unusual rheumatic illness, resulting in severe multi-system disease and premature death.Case report-Case descriptionA 69 year-old male presented to rheumatology and haematology with symmetrical polyarthritis, thrombocytopenia (18 x 109/L), eosinophilia (25.4 x 109/L), raised C-reactive protein (CRP, 43 mg/L), positive rheumatoid factor (>200), antinuclear antibody (ANA) and anti-Ro. Bone marrow biopsy did not demonstrate evidence of haematological malignancy.Seropositive rheumatoid arthritis and connective tissue disease overlap were diagnosed, and treatment with Prednisolone 60mg daily was initiated. Despite rituximab and intravenous immunoglobulins, thrombocytopenia deteriorated on reducing corticosteroids, however the addition of mycophenolate mofetil (MMF) allowed gradual prednisolone tapering to 3mg daily. Hydroxychloroquine was briefly added but discontinued due to headaches. MMF was discontinued after he developed fungal pneumonia followed by jaundice. Liver biopsy was consistent with drug-induced cholestasis, attributed to co-amoxiclav, and his liver function tests (LFTs) improved on ursodeoxycholic acid. Following a further deterioration in thrombocytopenia, hyperferritinaemia and new onset erythema nodosum, he had a repeat bone marrow examination. This demonstrated large areas of fibrosis and granulomatous inflammation with a dense, pleomorphic T-cell infiltrate, but no haemophagocytosis. Haematologists felt this was reactive and prednisolone dose was increased to 10mg daily.Six months later he developed cholangitis. Magnetic resonance cholangiopancreatography (MRCP) demonstrated a tight 4cm stricture of the distal common bile duct (CBD) within the head of pancreas, which was diffusely swollen without any clear focal mass. Serum amylase was mildly elevated (316 units/L). Concurrent CT thorax, abdomen and pelvis demonstrated bilateral ground-glass changes within the lungs, and a SARS-CoV-2 nasopharyngeal PCR test was positive, though he had no respiratory symptoms or oxygen requirement at that stage.Sadly, four days after the CT scan and before a planned endoscopic retrograde cholangiopancreatography (ERCP) could be performed, he became markedly hypoxic with plain chest X-ray features suggestive of COVID-19 pneumonia. Despite medical management, including doubling of his prednisolone dose, he rapidly deteriorated and died.Case report-DiscussionThis case highlights an unusual presentation of COVID-19 in a patient with a complex background of inflammatory arthritis with immune-mediated thrombocytopenia. At the time of his final illness, these conditions were managed with steroid monotherapy. Based on the COVID-19 risk matrix recommended by the British Society for Rheumatology, he was not identified as a patient requiring shielding.Cholangitis was the major problem precipitating his final admission to hospital, and at the time of admission he had no respiratory symptoms. One week prior to this admission, his father-in-law had died of COVID-19 pneumonia, though they had not been in recent direct contact. Interstitial lung changes were incidentally noted on a CT performed to identify the cause of cholangitis, which prompted the nasopharyngeal PCR that detected SARS-CoV-2. This occurred prior to widespread routine testing of hospital inpatients for SARS-CoV-2 by PCR. Unfortunately he then rapidly developed COVID-19 pneumonia and died before the underlying cause of cholangitis could be definitively identified, though an MRCP demonstrated an obstructed CBD within a diffusely swollen pancreas, where a differential diagnosis of pancreatic malignancy or autoimmune pancreatitis was suggested by the reporting radiologist.There are emerging case reports of COVID-19 resulting in significant pancreatic injuryand a further recent laboratory analysis has suggested that ACE2 receptors, which are utilised by SARS-CoV-2 to gain entry to host cells, are highly expressed on cholangiocytes at a comparable level to type II alveolar cells. Whilst the ultimate cause of cholangitis will remain unknown in this patient, this case highlights the potential for atypical presentations and extra-pulmonary manifestations of COVID-19.Case report-Key learning points COVID-19 is a multi-system illness which can cause significant extra-pulmonary as well as pulmonary pathology, with emerging reports that the biliary tract and pancreas are frequently affected.Evidence to inform accurate prediction of which patients with rheumatic diseases are at highest risk of acquiring severe COVID-19 disease remains insufficient, with current shielding guidelines based on expert consensus.This case highlights the importance of widespread testing for COVID-19 in hospital patients, as not all patients carrying the SARS-CoV-2 virus will demonstrate classical respiratory features of the disease at the point of admission.